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Aim: To estimate the role of different dysplastic syndromes and phenotypes in the development of sclerodegenerative lesions and calcific stenosis of the aortic valve (AV)., Subjects and Methods: One hundred and fifty patients (82 men and 68 women) aged 42 to 83 years were examined. A study group consisted of 89 patients (mean age 60.7 +/- 8.9 years) with clinical and echocardiographic signs of sclerodegenerative AV lesions (SDAVL). A control group included 61 patients (mean age 61.3 +/- 7.6 years) who had no clinical, physical, or echocardiographic signs of SDAVL. Twenty-five patients with critical calcific aortic stenosis (CAS) underwent histological examination of removed aortic semilunar valves after prosthetic AV replacement., Results: Persons with a Marfanoid habitus (18%) and mitral valve prolapse (10.7%) are most common in older age groups. An association has been found between the bone signs of dysmorphogenesis and the development of SDAVL and CAS., Conclusion: Three or more signs of bone dysmorphogenesis and the Marfanoid habitus should be viewed as a predictor of SDAVL.

Cardiovascular continuum in Marfan syndrome and clinically similar manifestations of inherited connective tissue pathology (ICTP) is discussed. New approaches to diagnostics and treatment of Marfan syndrome in conformity with revised Ghent criteria are described, the role of systemic involvement of connective tissue is discussed with reference to the relationship between transient cardiovascular continuum and complications of Marfan syndrome related to progression of aortic aneurysm and cardiomyopathy. The existence of dysplastic cardiomyopathy underlain by systemic involvement of connective tissue is postulated. The second part of the lecture deals with spatial phenotypic continuum the understanding of which is indispensable for diagnostics of ICTP. Marked variability of the signs of dysembryogenesis, its sensitivity and specificity are emphasized. It is concluded that connective tissue dysplasia can not be diagnosed based only on a few signs of dysembryogenesis.

Changes in activity of the components of TGF-β signaling pathway is associated with inherited disorders of connective tissue such as Marfan syndrome, Loeys-Dietz syndrome, etc. However, its impact on mitral valve prolapse (MVP) has not been completely studied. We examined 35 patients undergoing reconstructive surgery due MVP complicated by severe mitral insufficiency (mean age 62.5+/-7.9 years, 46% - men). High level of TGF-βl/2 was detected in majority (65%) of cases and correlated with the thickness of posterior leaflet (r=0.67; p=0.016), residual valve prolapse (r=0.68; p=0.007) and residual mitral regurgitation (MR) (r=0.56; p=0.01). In patients with high TGF-βl/2 level we detected a significant decrease in left ventricular longitudinal systolic (-13.5+/-2.2% vs. -16.6+/-2.3%, p=0.008) and diastolic (1.14+/-0.20 s-1 vs. 1.34+/-0.18 s-1, p=0.04) strain and SR (-0.89+/-0.15 s-1 vs. -1.14+/-0.15 s-1, p=0.002). Thus, TGF-β has a significant impact on the progression of valve myxomatous degeneration. The high activity of TGF-β signaling pathway results also in reduction in LV function, probably due to the profibrotic activity.

Aim: To investigate size of the aortal root and its valves in young patients and correlation between tricuspid aortal valve (TAV) asymmetry and connective tissue dysplasia (CTD)., Material and Methods: TAV symmetry was studied in young patients. Morphological examination consisted in phenotypic study of 34 corpses of young people who had died of noncardiological causes with a focus to internal signs of CTD signs and measurements of TAV and each cusp. Echocardiographic examination comprised phenotypic, clinical and echocardiographic studies of 144 young patients., Results: Echocardiographic diagnostic criteria of TAV asymmetry were specified. They consisted in eccentricity of the line of diastolic closure of aortal cusp (M-mode echocardiography) > 1.2; distinct differences in the size of the cusps (two-dimentional study), shift of at least one of the comissures by I hour at the "clock-face". A close correlation was detected between TA V asymmetry, external and clinical manifestations of CTD. The morphological criterion of TA V asymmetry--a more than 38% increase in the ratio of cusp area to sectional area of aortal root., Conclusion: TAV asymmetry is detected in 20% young people. It is closely related to external signs and clinical manifestations of CTD, other minor heart anomalies and may be accompanied with aortal regurgitation.

Twenty-two patients, aged 16 to 35 years who were to be operated on for varicocele have been examined. It has been established that the degree of shifts in hormonal homeostasis and central hemodynamics depends on the baseline level of physical training. Analysis of central hemodynamic response to operation stress has shown that the prognosis of favourable operation stress tolerance was observed in well trained patients or in those poorly trained without hyperkinetic hemodynamic reaction 1 day before surgery. The above data make it possible to predict the nature of reactions in response to the operation stress by the level of circulation function activation 1 day before surgery.

The first derivative of the integral tetrapolar rheogram of the body, body impedance recording, was found to show changes in blood flow in the arterial compression chamber. Evidence was given for a close relation of the distance between the electrodes while recording an integral rheogram of the body and the examinees' height. The relation of the distance to the examinees' height was equal in males and females and proved to be lower than that previously applied. For calculation of the stroke blood volume from the body impedance recording, the Kubicek equation was proposed to be used by introducing the height of an examinee instead of the distance between measuring electrodes. There was a good coincidence of results while calculating the stroke volume by impedance recording and Doppler echocardiography.

The elaboration of a standardized language for expressing the ECG diagnosis is of great practical importance and is necessary for the creation of a system of automatic treatment of the electrocardiosignal. A language is suggested for the ECG findings according to the changes in the ECG contour and disorders of the cardiac rhythm and conduction. It is based on the syndromal approach which makes it possible in analysis of the ECG to only distinguish definite syndromes without dictating a narrow diagnostic interpretation on the doctor. A list of 56 ECG diagnoses according to the character of the ECG contour and 60 elementary diagnoses relating to disorders of cardiac rhythm and conduction is given. The suggested language embraces all clinically important ECG syndromes and may be used in the elaboration of a system of automatic analysis and accepted as the foundation of a standardized language for expressing the ECG findings in practical electrocardiology.

The authors examined 1293 sportsmen engaged in different types of sporting activity and of a different level of mastership in their line and 482 young men who never went in for sports. They were subjected to electrocardiographic investigations with continuous recording of 100 cardiac cycles. The ECG analysis showed that sportsmen have disorders of cardiac rhythm in a statistically significant higher proportion of cases. The depressed activity of the sinus pacemaker is especially frequent which shows "arrhythmias of depressed sinus node" and extrasystoles. The latter in sportsmen are due to pathological changes related to the foci of chronic infections, myocardial dystrophy due to chronic physical overexertion and marked myocardial hypertrophy.

An attempt was made to reveal the possible relationship between ECG changes and the isotonic type of myocardial hyperfunction observed in sportsmen training to develop their endurance. Two groups of sportsmen were studied. Group 1 included 45 sportsmen of a high qualification developing their endurance. Group 2 consisted of control sportsmen with other trends in their training. The obtained results demonstrated that Group 1 sportsmen developed a significantly higher incidence of partial right bundle branch block and syndrome of delayed excitation of the right supraventricular crest, than Group 2 sportsmen. They also had a significantly higher amplitude of Q, R and T-waves and local electronegative period in the left thoracic leads. This permitted to conclude that the isotonic type of hyperfunction of the right or left ventricles in sportsmen training for higher endurance is reflected in their ECG.

The results of stroke volume determination by means of integral tetrapolar body rheography (ITBR) and M-echocardiography were compared in 78 individuals, while Doppler cardiography was used as the reference method. Fairly high accuracy of stroke volume measurement by ITBR and its advantages over echocardiography, particularly the possibility of calculating the stroke volume irrespective of the heart size, are demonstrated. The accuracy of the ITBR determination declines in patients with severe heart failure.

The authors analyzed the cause of diagnostic errors due to incorrect ECG interpretation. It was revealed that the cause for diagnostic errors may lie in the inadequate experience of the electrocardiologist , the overestimation of the method potentialities and the absence of the commonly accepted language of ECG interpretation. The necessity is emphasized of the clear cut differentiation between the electrophysiological and clinical-morphological approaches to the formation of the language of ECG interpretation. The clinical-morphological approach in clinical practice is acceptable only provided that the electrocardiologist is acquainted with the clinical picture of the disease. The use of this approach disregarding the clinical findings constitutes one of the causes of diagnostic errors and iatrogenia .

At present the work of automatization of the processes of the ECG analysis for diagnosing myocardial infarction with the help of computer technique is going forward on an ever broader scale. For an automatic identification of the focal changes syndrome on the ECG, which in the clinical practice is considered to be tantanount to myocardial infarction, the use of a special diagnostic device (SDD) is proposed. The SDD are comparatively cheap and simple in operation. The process of diagnosing provides for utilization of medical experience, but, at the same time, eliminates shortcomings inherent in systems that reproduce the physicians' manipulations in establishing the diagnosis of myocardial infarction with the help of an electrocardiogram. The choice of the SDD was made by stimulating it with an all-purpose computer. The results of clinical trials of the SDD conducted at specialized clinics bear proof to great possibilities of the automatic electrocardiographic diagnostics.

The syndrome of mitral valve prolapse was diagnosed by means of echocardiography in 11 of 28 top-class athletes with functional systolic murmurs and in 4 of 16 athletes examined irrespective of the physical findings and complaints. The echocardiographic manifestations of the prolapse consisted in delayed systolic flexion of the posterior cusp (3 cases), early systolic prolapse of the posterior cusp (4) and holosystolic prolapse of both cusps (8). It is concluded that the syndrome of mitral valve prolapse occurs in athletes far more often than generally supposed and may be the cause of systolic murmurs, and the development of myocardial dystrophy due to chronic physical overstrain and disorders of cardiac rhythm.